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Atypical teratoid rhabdoid tumors of the posterior fossa in children

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Abstract

Purpose

Atypical teratoid rhabdoid tumors (AT/RT) are rare, aggressive, central nervous system neoplasms that typically affect children under 3 years of age and have a very poor prognosis. Early case series consistently demonstrated rapid recurrence with progression to death, but more recent experience has shown significant improvements in progression free and overall survival.

Methods

A retrospective analysis of the clinical data of children diagnosed with AT/RT at the Ann & Robert H. Lurie Children’s Hospital of Chicago (formerly Children’s Memorial Hospital) between 2000 and 2014 was performed. Overall survival (OS) was used to describe outcome. Our small sample size and the utilization of different adjuvant regimens over the study period precluded a detailed statistical analysis.

Results

Eight children with AT/RT of the posterior fossa were included in our report. Gross total resection (GTR) was achieved in five children (63 %), two children underwent subtotal resection (25 %), and there was one who underwent biopsy. Patients were treated with various combinations of chemotherapy with or without conformal radiation therapy (RT). Median overall survival was 5 months (range 1 to 107 months) with two patients achieving sustained responses to 45 and 107 months.

Conclusions

Our experience is in line with prior reports that show that children diagnosed with AT/RT of the posterior fossa have a poor prognosis, but that long-term survival is possible. These tumors provide many challenges, but contemporary series are beginning to show improvements in survival.

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Conflict of interest

The authors have no conflicts of interest to disclose.

Author’s contributions

Arthur J. DiPatri, Jr., MD

Conception and design, acquisition of data, analysis and interpretation of data, drafting the article, critical revision of the article, and final approval of the version to be published

Simone Treiger Sredni, MD, PhD

Drafting the article, critical revision of the article, and final approval of the version to be published

Tadanori Tomita, MD

Conception and design, analysis and interpretation of data, critical revision of the article, and final approval of the version to be published

Gordan Grahovac, MD

Acquisition of data and final approval of the version to be published

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Authors and Affiliations

  1. Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Arthur J. DiPatri Jr., Simone Treiger Sredni & Tadanori Tomita

  2. Ann & Robert H. Lurie Children’s Hospital of Chicago Chicago, Division of Pediatric Neurosurgery, 225 E. Chicago Avenue, Box 28, Chicago, IL, 60611-2605, USA

    Arthur J. DiPatri Jr., Simone Treiger Sredni, Gordan Grahovac & Tadanori Tomita

  3. Cancer Biology and Epigenomics Program, Stanley Manne Children’s Research Institute, Chicago, IL, USA

    Simone Treiger Sredni

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  1. Arthur J. DiPatri Jr.
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DiPatri, A.J., Sredni, S.T., Grahovac, G. et al. Atypical teratoid rhabdoid tumors of the posterior fossa in children. Childs Nerv Syst 31, 1717–1728 (2015). https://doi.org/10.1007/s00381-015-2844-x

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